Maximizing Treatment Effects with VPI and Cleft Lip and Palate

Scott Prath, M.A., CCC-SLPKeith Lebel, M.A., CCC-SLP

Photos courtesy of Operation Smile

TSHA Annual Convention March 9th, 2012- San Antonio, Texas

Structural Anomalies

Learner Objectives

•Describe how craniofacial differences occur and how they negatively impact intelligibility

•List underlying speech components that can be affected by VPI and Cleft Lip and/or Palate

•Identify intervention and evaluation strategies for working with a child with craniofacial anomalies

Defining VPI and VPD

•Velo-pharyngeal Insufficiency▫The velum (velo) is not contacting the

pharynx (back wall of the throat)▫Can be caused by:

Muscle weakness A large opening Insufficient muscle function Adenoids and tonsils

Defining VPI and VPD

• Velo-pharyngeal Dysfunction (VPD)▫ Increasingly VPD is being used as the umbrella

term as VPI can be confusing. ▫VPD does not exclude symptoms:

Anatomic Myoneural Behavioral Combinations of disorders

▫VPD includes: Velopharyngeal insufficiency (lack of closure) Velopharyngeal incompetence Velopharyngeal inadequacy Velopharyngeal incorrect learning

What is a Cleft?

• Clefts result from incomplete development of the lip and/or palate in the early weeks of pregnancy.

What types of clefts exist?

• A cleft lip (CL) is a separation in the upper lip.

• A cleft palate (CP) is an opening in the roof of the mouth.

• A cleft lip and palate (CLP) extends through both.

What types of cleft palates exist?

• A cleft palate can be:

▫ unilateral

▫ bilateral

▫ submucousal with bifid uvula

What is the prevalence?

• Clefts occur in ~ 1/750 live births

• Estimated prevalence by type:Lip only 14%Palate only 37%Lip and Palate 49% (77% are unilateral left)

• Estimated data by race:▫ Asian and Caucasian populations more susceptible▫ Black populations present with ½ the rate of Asians

and Caucasians▫ Hispanic data are inconclusive

When does a cleft occur?

4 and 5 weeks in utero

When does a cleft occur?

5 ½ and 6 weeks in utero

When does a cleft occur?

7 and 8 weeks in utero

When does a cleft occur?

What causes a cleft?

1. Gene Mutation2. Chromosomal Aberrations3. Teratogenic Agents4. Multifactoral Inheritance5. Mechanical Factors

How is a cleft repaired?

The repair of a cleft lip or palate is the process of taking

existing tissue that surrounds

the opening and rotating it or

moving it to close the opening.

What is the process for the family?

Influences on family effectiveness

Parent Reaction to a CleftChild Reaction to a CleftParent InvolvementMother-child Bond and Feeding

What is the process for the family?The Cleft Palate Team• Clinic Director• Audiologist• Craniofacial Surgeon• Geneticist• Neurosurgeons• Nurses• Occupational Therapists• Oral and Maxillofacial

Surgeons• Orthodontists• ENTs• Pediatrician• Pediatric Anesthesiologists

• Pediatric Dentists and Prosthodontists

• Pediatric Radiologists• Physical Therapists• Plastic Surgeons• Speech Language

Pathologists• Surgeon’s Assistant

Socio-emotional Development

“It is reasonable to assume that individuals with CLP should have relatively normal long-term health, as CLP is a reparable birth defect. However, several studies have suggested that individuals with CLP have a higher than expected incidence of psychiatric and behavior diseases, an increased risk for cancer, and increased mortality…”

Southern medical journal volume 99 number 11 oct 06 p 1112, Robin, Bates, etc.

The effects of CLP on the parent

Effects on the parent▫Blame due to improper love, nutrition, an

event during pregnancy▫Parent’s personality and their feeling of

control or lack of control▫Feelings of disappointment or resentment if

they feel that this event has dashed their hopes

The effects of CLP on the parent

Intervention:▫ Education!▫ Support!Research shows that when a family is in a

position where they feel that personal needs or aspects of their lives are governed by external forces or in a poor state, they exhibit negative feelings toward their child or event.

The effects of CLP on the child

Effects on the child▫Speech or hearing disorders▫Parents’ feeling of guilt

Can cause a parent to treat a child differently▫Anxiety

Regular or protracted doctor visits Parents’ ever-present fear of choking

▫Visible scars due to surgical procedures

The effects of CLP on the child

Intervention:The child’s psychosocial state is dependent

on how well or poorly the parent is dealing with everything.

It is our role to support and improve the lives of the child. This means direct social intervention with the parent.

When do emotions set in?

•Periods of: Anxiety Optimism Depression Acceptance

occur with each surgery or event.Edwards and Watson, 1980, found that there is an

optimistic period right after birth because the couple is happy that there are surgical options available, but soon after they can become disheartened.

•Take home message:Work to identify how a family is dealing with the process

in the time that we are serving them.

The Kubler-Ross Grief Cycle•Denial: Example - “She’s fine."; "This can't be

happening."

•Anger: Example - "Why me? It's not fair!" "NO! NO! How can you accept this!"

•Bargaining: Example - "Just let him talk fine, I don’t mind the scar."

•Depression: Example - "I'm so sad, why bother with anything?"; “Everything that we had planned for her isn’t going to happen."

•Acceptance: Example - "It's going to be OK."; "I can't fight it, I may as well prepare for it."

Parent Involvement

•Andhra Pradesh, India▫Tested:

Known context (rhymes, counting 1-10) Unknown context (family information)

▫There was a greater understandability of unknown contexts after treatment

Feeding and psychological development

•Children gain pleasure from oral stimulation and feeding

Feeding is impaired Feeding issues are one of the first problems

that families encounter so it is not an enjoyable event

•The early maternal-bonding process is often more disturbed by feeding problems than by the facial defect

Kalland, 1995

Feeding and psychological development

•CL (cleft lip only) -usually no major feeding problem (breast feeding possible)

•CP has difficulty feeding because of inability generating effective oral suction

Feeding •Children with CLP take in more air

▫They need to be burped more and fed more upright

•Feedings should last ~30 minutes.▫If they are needing 40 minutes, enlarge

and/or cross cut the nipple opening•Weight gain, feeding frequency, and

feeding amount are normally the same for a child with CLP

How is communication affected?

Speech DevelopmentArticulationVoiceResonance

Language DevelopmentHearing Development

Communication Characteristics

Children with CLP:•Show deficits in the size and

composition of their early sound inventories

•Reach the babbling stage later •Show less variety of speech forms

produced•Have a hypo-or hyper-nasal quality

Communication Characteristics

Children with CLP:•Often have voice problems (harshness)

due to overuse•Can have constant ear infections and

temporary hearing loss•Do not necessarily have cognitive

issues

Outcomes are influenced by severity and time of surgery

Language Development

•Study using the TOLD-P (63, 4-5 y.o.)▫21% manifested receptive impairments▫26% manifested expressive impairments▫General Population

3.3%•Take home message

▫Treat CLP as a comprehensive communication disorder, not just a speech disorder

Hearing development

Type of loss 4-5 years 14-15•WNL (0-26 dB) 78 88•Mild (2-40 dB) 22

12

22% exhibit hearing loss (1/5)

•Take home message▫Keep current on hearing status. Hearing

loss = speech and language loss

Successful Diagnostics:Using the VPI Pyramid for a bottom-to-top approach

Underlying Motor AbilitiesPurposeful Use of Motor AbilitiesTypical Focus of a Standard Speech Assessment

VPI Pyramid

Vertical Jaw Movement

A child’s ability to produce the full spectrum of sounds is dependent on jaw height.

•Is a child using the full range of possible movement?

•How expressive is she with facial movements such as smiling?

Vertical Jaw Movement

VPI Pyramid

Breath Support

An utterance is dependent on breath support.

•Can a child independently inflate the lunges and diaphragm?

•Can he inflate both together?•How is his posture?•Are you doing therapy in a chair or on the

floor?

Breath Support

VPI Pyramid

Anterior/Lateral Lip Movement

The sound repertoire is also dependent on manipulation of the oral chamber.

•Can the child produce:▫u-e▫a-o

Anterior/Lateral Lip Movement

VPI Pyramid

Tongue Placement

Consonant repertoires are dependent on tongue placement

•Can the child follow commands to:▫Touch the teeth▫Stick out her tongue

•Do consonants sound like their nasal pairs?▫/d/, /t/. /l/ = /n/▫/b/, /p/= /m/▫/g/, /k/, = “ng”

Tongue Placement

VPI Pyramid

Phonation

Differentiation between minimal pairs is dependent on the ability to control phonation.

•Can a child turn phonation on and off?•How long can she phonate for?

Phonation

VPI Pyramid

Imitation of a Movement

Therapeutic success is dependent on the child’s ability to imitate movements

•Does the child have experience following commands? (Simon says)

•Can they imitate articulator movements?

7/10

Imitation of a

Movement

Imitation of a Movement Imitation of a

Movement

VPI Pyramid

Imitation of a Vowel

•Vowels carry the message. The presence of vowels is something that we normally take for granted.

•Take a vowel inventory ▫Target vowels in isolation (a)▫In strings (a,a,a,a)▫In opposition (u-I, u-I a-o, a-o) 7/10

Imitation of a Vowel

VPI Pyramid

Imitation of a Consonant

Consonants require:▫Articuator movements (possibly tongue,

cheeks, and lips)▫Air manipulation (phonation or bursts)

•Can she imitate a consonant?•If not, what part of the lower pyramid is

she missing?

Imitation of a

Consonant

7/10

Imitation of a Consonant Imitation of a

Consonant

VPI Pyramid

Production of Sounds and Syllables

Syllable production is normally where therapy begins•Everything below on the pyramid needs to

be in place in order for “meaning” to occur

•The pyramid provides us with a lot of areas to focus on that are not dependent on surgeries

Production of

Sounds and

Syllables

Production of Sounds and Syllables

Production of

Sounds and

Syllables

Communication goals/ Outcomes for Structural Anomalies Increase Vowel Repertoire Increase Consonant Inventories Increase Vocabulary Increase Oral Airflow Decrease use of Nasal and Glottal Sounds

Increase Vowel RepertoireDate October 10th

a 10/10

e 3/10

i 0/10

o 10/10

u 4/10

a-o 0/10

u-e 0/10

Date October 10th November 15th

a 10/10

e 3/10 5/10

i 0/10 3/10

o 10/10

u 4/10 8/10

a-o 0/10 4/10

u-e 0/10 4/10

Date October 10th November 15th December 7th

a 10/10

e 3/10 5/10 10/10

i 0/10 3/10 7/10

o 10/10

u 4/10 8/10 10/10

a-o 0/10 4/10 6/10

u-e 0/10 4/10 7/10

Increase Vowel Repertoire

Increase Consonant Inventories

• Hi• Hello• Hey• Mommy• More• Me• No• Whoa• Wow• Honey

• Mamá• No • Mío• Niña• Niño• Ojos• En• Mano• Wawa – agua

BEFORE palate repairLOW pressure words to target

Increase Consonant Inventories

• Baby• Boy• Pop• Pooh• Pie• Toy• Doll• Daddy• Cookie• Go

• Papá• Bebé• Boca• Gato• Todo• Tú• Tío• Qué• Ten

AFTER palate repairHIGH pressure words to target

Increase Consonant Inventories

Increase Vocabulary

•Sounds and vocabulary develop in tandem•Do we:

▫Focus on articulation to give her the sounds to produce more language?

▫Focus on language to give her a way to practice her sounds?

Any ideas?

Increase Vocabulary

•Vocabulary development should be targeted with sound development▫Choose words that:

Are common and in their environment Are useful Are extremely fun(read: routines-based intervention)

•Syllable should be simple CV (consonant/vowel)

•Start with stops and bilabial sounds

Increase Vocabulary

Increase Oral Airflow

•A child with a cleft does not have control over the air leaving their throat

•In typical development we stop or slowly release this air to produce speech

•Regardless of what surgeries a child has undergone, we need to familiarize the child with airflow through the mouth

Increase Oral Airflow

•Request an easy repetition (muh,muh,muh)▫After the child starts repeating, plug his

nose•Inhale deeply, hold your breath, and

explode out with a single sound▫BUH!, PUH!

Increase Oral Airflow

Decrease use of Nasal and Glottal Sounds

13% of 63, 4-5 year olds presented with some form of voice disorder▫harshness, breathiness, nodules

• Growls and nasal sounds are typical for young infants but they are used less when consonants develop

• Children with clefts retain these sounds• Parents, wanting communication, reinforce

these sounds

Decrease use of Nasal and Glottal Sounds

•Acknowledge the child’s attempt but then requests other consonants or sounds

•Pair voiceless consonants with whispered vowels

puh/tuh/ku/huh•This keeps the glottis open and prevents

the glottal stop from occurring

Case StudyApplication to Academic Needs

Identification of Initial SoundWords Answers 09/29/2011

Tobillo /t/ 0

Zorro /s/ 1

Humano /u/ 0

Diamante /d/ 0

Planta /p/ 0

Jamon /j/ 1

Lluvia /y/ 0

Restaurante /r/ 0

TOTAL 2

01/24/2012

0

1

1

1

1

1

1

1

7

Identification of Final SoundWords Answers 09/29/2011

Entro /o/ 1

Tribu /u/ 1

Fotos /s/ 1

Nueve /e/ 1

Album /m/ 0

Estrella /a/ 1

Calidad /d/ 1

Caminar /r/ 0

TOTAL 6

01/24/2012

1

1

1

1

0

1

0

0

5

Identification of Final Sound

Union of SoundsWords Answers 09/29/2011

/t/ /e/ /ch/ /o/ Techo 0

/j/ /i/ /s/ Gis 1

/c/ /a/ /y/ /e/ Calle 1

/y/ /e/ /m/ /a/ Yema 0

/b/ /u/ /rr/ /o/ Burro 0

/v/ /e/ /l/ /o/ /s/ Veloz 0

/p/ /l/ /u/ /m/ /a/ Pluma 0

/r/ /e/ /g/ /l/ /a/ Regla 1

TOTAL 3

01/24/20121

0

1

1

1

1

1

1

0

6

Union of Sounds

Segmentation of SoundsWords Answers 09/29/2011

Ola /o/ /l/ /a/ 1

Mesa /m/ /e/ /s/ /a/ 0

Ella /e/ /y/ /a/ 1

Bello /b/ /e/ /y/ /o/ 0

Pina /p/ /i/ /ny/ /a/ 0

Jabon /j/ /a/ /b/ /o/ /n/ 0

Arbol /a/ /r/ /b/ /o/ /l/ 0

Verde /v/ /e/ /r/ /d/ /e/ 0

TOTAL 2

01/24/2012

0

1

1

1

1

1

0

0

5

Segmentation of Sounds

Omission of Initial SoundWords Omission Answers 09/29/201

1

Les /l/ (es) 1

Cama /k/ (ama) 0

Rojo /r/ (ojo) 1

Llave /y/ (ave) 1

Marco /m/ (arco) 0

TOTAL 3

01/24/2012

1

1

1

1

0

5

Omission of Initial Sound

Omission of Final SoundWords Omission Answers 09/29/201

1

Osa /a/ (os) 1

Solar /r/ (sola) 0

Hacen /n/ (hace) 1

Capaz /s/ (capa) 0

Pared /d/ (pare) 1

TOTAL 3

01/24/2012

1

1

0

1

1

4

Omission of Final Sound

Recognizing WordsWords 09/29/2011

Luz 0

Vive 0

Lleva 0

Voy 0

Hay 0

Tortuga 0

Caballo 0

Ensena 0

Hombres 0

Chocolate 0

Alfombra 1

Pequenos 1

TOTAL 2

01/24/20121

1

1

0

1

1

1

1

0

0

0

0

0

6

Recognizing Words

VPI Pyramid

Resources

•www.cleft.com•1-800-24CLEFT•www.operationsmile.org•www.widesmiles.org •The story of Lippy the Lion•The story of Thumper, the Cleft

Affected Bunny•www.bilinguistics.com

More Great Resources onCultural and Linguistic Diversity

A word from the parents

Parents in one study reported feelings of anxiety about how the baby would be able to eat.▫Set therapy goals to address feeding▫Work with nutritionists and the cleft palate

team

A word from the parents

Parents DID report positive feelings from professionals who did not ignore condition.▫Do not ignore the condition

A word from the parents

Many expressed desire to meet other parents of a child with CL/CP—to exchange thoughts and discuss practical problems.▫Seek out support groups, resources, or

other families to network with

A word from the parents

Negative reactions from other people were perceived through body language, such as keeping at a distance, looking away.▫Be aware of your body language as well

when interacting with child and family

A word from the parents

Parents often hear “He will be fine later” from staff and other professionals and interpreted this to mean that child was not considered fine at that moment.▫Be supportive and concerned about the

current condition▫Parents stated that they grew

tired of hearing these phrases

A word from the CLP teamWhat would you consider to be the most

important thing that a service provider should know when they are working with a child with VPI or a cleft lip and palate?

Kids with clefts can’t generate pressure to make sounds. Normally they just say “muh.” We need to brush up on our A&P so that we can provide good therapy.

A word from the CLP team

What should professionals educate parents on?

The second surgery normally occurs around 12 months so kids with clefts won’t develop speech sounds on time. Let the parents know that they should be looking for and practicing low pressure sounds to get the ball rolling.

A word from the CLP team

Is there anything that the parents could know from service providers to make the team and surgery visits easier?

Keep the child’s hearing status up to date. Hearing issues like infections can delay speech even further and cause schedules to be pushed back or visits to be cancelled.

A word from the CLP team

Is there anything problematic/difficult with family interactions that could be addressed by services outside of the team visits?

You need to do a full assessment to determine whether there are cognitive delays or other delays. Parents fear that their children are delayed in every way imaginable because of the cleft. Normally communication is the only, main issue.

Thank you!